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Journal of Cardiac Surgery Jul 2020Tricuspid atresia (TA) is a complex congenital heart disease that presents with cyanosis in the neonatal period. It is invariably fatal if left untreated and requires... (Review)
Review
Tricuspid atresia (TA) is a complex congenital heart disease that presents with cyanosis in the neonatal period. It is invariably fatal if left untreated and requires multiple stages of palliation. Early recognition and timely surgical intervention are therefore pivotal in the management of these infants. This literature review considers the pathophysiology, presentation, investigations, and classification of TA. Moreover, it discusses the evidence upon which the latest medical and surgical treatments are based, as well as numerous recent case reports. Further work is needed to elucidate the etiology of TA, clarify the role of pharmacotherapy, and optimize the surgical management that these patients receive.
Topics: Alprostadil; Angiotensin-Converting Enzyme Inhibitors; Child, Preschool; Female; Fontan Procedure; Humans; Infant; Infant, Newborn; Male; Postoperative Care; Survival Rate; Treatment Outcome; Tricuspid Atresia; Tricuspid Valve
PubMed: 32484582
DOI: 10.1111/jocs.14673 -
Current Problems in Cardiology Apr 2022
Review
Topics: Humans; Persistent Left Superior Vena Cava; Pulmonary Arterial Hypertension; Tricuspid Atresia; Vena Cava, Superior
PubMed: 34020773
DOI: 10.1016/j.cpcardiol.2021.100837 -
Journal of Cardiac Surgery Jul 2022Tricuspid atresia with an absent pulmonary valve is a rare congenital cardiac defect. Although extensive pathological reviews have been published in the past, there are...
Tricuspid atresia with an absent pulmonary valve is a rare congenital cardiac defect. Although extensive pathological reviews have been published in the past, there are only a handful of cases that have been successfully palliated to the stage of Fontan. We hereby describe the successful management of one such case and review the surgical strategies described in the literature.
Topics: Fontan Procedure; Humans; Pulmonary Atresia; Pulmonary Valve; Tricuspid Atresia
PubMed: 35434814
DOI: 10.1111/jocs.16535 -
British Heart Journal Oct 1956
Topics: Humans; Tricuspid Atresia; Tricuspid Valve
PubMed: 13374157
DOI: 10.1136/hrt.18.4.499 -
British Medical Journal Nov 1957
Topics: Humans; Tricuspid Atresia; Tricuspid Valve
PubMed: 13479699
DOI: 10.1136/bmj.2.5056.1283 -
Cardiology in the Young Jul 2023Tricuspid atresia with common arterial trunk is a very rare association in complex CHD. This association has even more infrequently been documented concomitantly with...
Tricuspid atresia with common arterial trunk is a very rare association in complex CHD. This association has even more infrequently been documented concomitantly with interrupted aortic arch. We present the diagnosis and initial surgical management of an infant with a fetal diagnosis of tricuspid atresia and common arterial trunk, with additional postnatal finding of interrupted aortic arch with interruption between the left common carotid and left subclavian artery. Due to the infant's small size, she was initially palliated with bilateral pulmonary artery bands and a ductal stent. This was followed by septation of the common arterial trunk and interrupted aortic arch repair and 4 mm right subclavian artery to main pulmonary artery shunt placement at two months of age. She was discharged home on day of life 81.
Topics: Infant; Female; Humans; Tricuspid Atresia; Truncus Arteriosus, Persistent; Pulmonary Artery; Aorta, Thoracic
PubMed: 36472122
DOI: 10.1017/S1047951122003602 -
Cardiology in the Young Mar 2022We report a neonate with dilated cardiomyopathy and have echocardiographic findings consistent with "functional" tricuspid atresia. There was an echo-bright, plate-like...
We report a neonate with dilated cardiomyopathy and have echocardiographic findings consistent with "functional" tricuspid atresia. There was an echo-bright, plate-like tissue at the tricuspid valve position with no forward flow across it. This report underscores the role of right ventricle intracavitary haemodynamic influence on the tricuspid valve leaflet excursion and demonstrates a phenomenon of "pseudo or functional tricuspid atresia" mimicking tricuspid atresia in a patient with acute presentation of cardiomyopathy.
Topics: Cardiomyopathy, Dilated; Echocardiography; Heart Ventricles; Humans; Infant, Newborn; Pulmonary Atresia; Tricuspid Atresia; Tricuspid Valve
PubMed: 34350823
DOI: 10.1017/S104795112100322X -
Neonatal Network : NN Jul 2017Tricuspid atresia (TA) is a rare congenital heart defect in which the right atrioventricular connection, the tricuspid valve, is absent. As a result, there is no direct... (Review)
Review
Tricuspid atresia (TA) is a rare congenital heart defect in which the right atrioventricular connection, the tricuspid valve, is absent. As a result, there is no direct communication between the right atrium and right ventricle. Surgical treatment, including the Fontan procedure, is indicated yet palliative, leaving patients with various lifelong complications. A comprehensive literature review revealed a paucity of evidence-based education on the identification, evaluation, management, treatment, and life span implications of TA. We present a case of TA from birth through adulthood, while simultaneously assessing the literature, to report the most current evidence relative to living with TA after surgical palliation. In addition, the embryology, methods of prenatal and postnatal diagnosis, potential complications, management, anticipatory guidance, and educational needs of both parents and patient are discussed.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Education, Nursing, Continuing; Female; Fontan Procedure; Heart Atria; Heart Ventricles; Humans; Infant; Infant, Newborn; Male; Middle Aged; Treatment Outcome; Tricuspid Atresia; Tricuspid Valve; Young Adult
PubMed: 28764825
DOI: 10.1891/0730-0832.36.4.218 -
Pediatric Cardiology Apr 2022Tricuspid valve agenesis/atresia (TVA) is a congenital cardiac malformation where the tricuspid valve is not formed. It is hypothesized that TVA results from a failure...
Tricuspid valve agenesis/atresia (TVA) is a congenital cardiac malformation where the tricuspid valve is not formed. It is hypothesized that TVA results from a failure of the normal rightward expansion of the atrioventricular canal (AVC). We tested predictions of this hypothesis by morphometric analyses of the AVC in fetal hearts. We used high-resolution MRI and ultrasonography on a post-mortem fetal heart with TVA and with tricuspid valve stenosis (TVS) to validate the position of measurement landmarks that were to be applied to clinical echocardiograms. This revealed a much deeper right atrioventricular sulcus in TVA than in TVS. Subsequently, serial echocardiograms of in utero fetuses between 12 and 38 weeks of gestation were included (n = 23 TVA, n = 16 TVS, and n = 74 controls) to establish changes in AVC width and ventricular dimensions over time. Ventricular length and width and estimated fetal weight all increased significantly with age, irrespective of diagnosis. Heart rate did not differ between groups. However, in the second trimester, in TVA, the ratio of AVC to ventricular width was significantly lower compared to TVS and controls. This finding supports the hypothesis that TVA is due to a failed rightward expansion of the AVC. Notably, we found in the third trimester that the AVC to ventricular width normalized in TVA fetuses as their mitral valve area was greater than in controls. Hence, TVA associates with a quantifiable under-development of the AVC. This under-development is obscured in the third trimester, likely because of adaptational growth that allows for increased stroke volume of the left ventricle.
Topics: Echocardiography; Female; Fetal Heart; Humans; Pregnancy; Pulmonary Atresia; Tricuspid Atresia; Tricuspid Valve; Ultrasonography, Prenatal
PubMed: 34988599
DOI: 10.1007/s00246-021-02789-6 -
South African Medical Journal =... Oct 1964
Topics: Angiography; Cardiac Catheterization; Cardiac Surgical Procedures; Electrocardiography; Heart Defects, Congenital; Heart Septal Defects; Heart Septal Defects, Atrial; Humans; Pulmonary Circulation; Pulmonary Heart Disease; Thoracic Surgery; Tricuspid Atresia; Tricuspid Valve; Tricuspid Valve Stenosis
PubMed: 14212649
DOI: No ID Found